Table 1 Patient characteristics.
No of patients | 26 |
No of transplants | 26 |
Median age of transplant | 5 years (6 months-17.1 years) |
Female:Male | 8:18 |
Pretransplant diagnosis | |
Idiopathic severe aplastic anemia Thalassemia Inherited marrow failure syndrome Congenital amegakaryocytic thrombocytopenia Hemophagocytic lymphohistiocytosis Wiskott-Aldrich Syndrome DOCK8 deficiency CD40 ligand deficiency Severe Combined Immunodeficiency CTLA4 deficiency Leukocyte Adhesion Deficiency type 1 CSF2RA deficiency Severe congenital neutropenia | 5 4 2 2 3 2 2 1 1 1 1 1 1 |
Stem cell source | |
Peripheral blood | 26 |
Lansky score | |
100 90 80 < = 70 | 20 1 1 4 |
Conditioning regimens* | |
Fludarabine-Cyclophosphamide Fludarabine-Treosulfan Fludarabine-Treosulfan-Thiotepa Fludarabine | 2 8 15 1 |
Alemtuzumab from day-6 to day-4 | |
0.5 mg/kg 0.9 mg/kg | 14 12 |
GvHD prophylaxis | |
Ciclosporin and mycophenolate mofetil | 26 |
Donor Characteristics | |
10/10 HLA-matched Family donor (Sibling = 10; Parent = 4) Matched Family donor with 9/10 bidirectional mismatch Matched Family donor with homozygous 8/10 in GvH direction Matched unrelated donor Matched unrelated donor with 9/10 homozygous allele in GvH direction | 14 1 1 8 2 |
Pre-transplant CMV status (recipient/donor) | |
pos/pos pos/neg neg/pos On IVIg/pos | 19 3 2 2 |
Pre-transplant EBV status (recipient/donor) | |
pos/pos pos/neg neg/pos neg/neg pos/not done neg/not done On IVIg/pos On IVIg/neg | 13 1 1 1 4 4 1 1 |
